Glanzmann's thrombasthenia associated with gastrointestinal angiodysplasias successfully treated with bevacizumab.

Glanzmann's Thrombasthenia (GT) is a rare hemorrhagic condition caused by a platelet surface receptor disorder of the glycoprotein (GP) IIb/IIIa. Symptoms of GT are various forms of hemorrhages, such as purpura, epistaxis and menorrhagia. Gastrointestinal bleeding (GIB) is a rare expression of the condition and may occur due to traumas in the GI tract or as a consequence of gastrointestinal angiodysplasia (GIADs). In this case report, we present a middle-aged woman with recurrent GIB consequent to GIADs with persistent melena and iron deficiency anemia. After several unsuccessful therapeutic interventions, the patient was studied by the hereditary hemorrhagic telangiectasia's (HHT - Osler-Weber-Rendu disease) unit, where she received bevacizumab, showing a complete improvement in symptoms as well as a reduction in her GIADs. This case shows that bevacizumab could be a possible line of treatment for patients with coagulation disorders with GIADs.

[Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia]. / Síndrome de Evans y artritis reumatoidea como manifestaciones autoinmunes de la leucemia de células T grandes granulares.

Large granular T-cell leukemia is a rare cytotoxic lymphocyte disorder. These cells play an integral role in the immune system and are divided into 2 lineages: CD3 T positive and natural killer. Its proliferation and uncontrolled cytotoxicity can generate autoimmunity or malignancy. Rheumatoid arthritis is the most common autoimmune disease in individuals with this type of leukemia, however, it has been associated with a wide spectrum of other autoimmune diseases and hematological conditions including hemolytic anemia, pure red blood cell aplasia, and neutropenia, leading to recurring bacterial infections. The following is a case of a 72-year-old female with a history of large granular T-cell leukemia and manifestations compatible with rheumatoid arthritis, which occurs with a severe Evans syndrome with a good initial and sustained response to gamma globulin, corticosteroid therapy, and rituximab.

La leucemia de células T grandes granulares es un trastorno poco frecuente de linfocitos citotóxicos. Estas células juegan un rol integral en el sistema inmunológico y se dividen en 2 linajes: T CD3 positivas y natural killer. Su proliferación y citotoxicidad descontrolada puede generar autoinmunidad o malignidad. La artritis reumatoide es la enfermedad autoinmune más común en individuos con este tipo de leucemia, sin embargo, se ha asociado a un amplio espectro de otras enfermedades autoinmunes y afecciones hematológicas incluyendo anemia hemolítica, aplasia pura de glóbulos rojos y neutropenia, que conducen a infecciones bacterianas recurrentes. Se presenta a continuación una paciente de 72 años con antecedentes de leucemia de células T grandes granulares y manifestaciones compatibles con artritis reumatoidea, que intercurre con un Síndrome de Evans grave con buena respuesta inicial y sostenida a gammaglobulina, corticoterapia, y rituximab.

Low Incidence of Symptomatic Thrombotic Events in Adult Patients Hospitalized with Coronavirus 19: A Retrospective Cohort Study.

BACKGROUND: Since the beginning of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) pandemic, there have been many reports of increased incidence of venous thromboembolism and arterial events as a complication. OBJECTIVE: To determine the incidence of symptomatic thrombotic events (TEs) in patients hospitalized for SARS-CoV2 disease (coronavirus 19 [Covid-19]). METHODS: A retrospective single-center cohort study with adult patients with a positive reverse transcriptase-polymerase chain reaction (rt-PCR) for SARS-CoV2, included from the date of diagnosis of Covid-19 and followed for 90 days or until death. RESULTS: A total of 1621 patients were included in this study. The median age was 73 years (interquartile range25th-75th [IQR] 53-87 years) and 57% (913) were female. Overall mortality was 21.6% (348). The overall incidence of symptomatic TEs within 90 days of diagnosis was 1.8% (30 of 1621) occurring in 28 patients, including an incidence of pulmonary embolism of 0.9% (15, 95% confidence interval [CI] 0.60%-1.6%), deep venous thrombosis of 0.61% (10, 95% CI 0.2%-1%), ischemic stroke of 0.25% (4, 95% CI 0.09%-0.65%), and ischemic arterial events of 0.06% (1, 95% CI 0.008%-0.43%). No acute coronary syndrome events were recorded. The incidence of symptomatic TEs was significantly lower in the general ward than in intensive care units (1.2% vs 5.7%; p < .001). The median time since positive rt-PCR for SARS-CoV2 to symptomatic TE was 22.5 days (IQR 19-43 days). There was no significant difference in the proportion of patients receiving (53.6%) and not receiving thromboprophylaxis (66.5%) and the development of TEs. CONCLUSION: The overall incidence of symptomatic TEs among these patients was lower than the incidence previously reported.

Síndrome de Evans y artritis reumatoidea como manifestaciones autoinmunes de la leucemia de células T grandes granulares / Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia

Resumen La leucemia de células T grandes granulares es un trastorno poco frecuente de linfocitos citotóxicos. Estas células juegan un rol integral en el sistema inmunológico y se dividen en 2 linajes: T CD3 positivas y natural killer. Su proliferación y citotoxicidad descontrolada puede generar autoinmunidad o malignidad. La artritis reumatoide es la enfermedad autoinmune más común en individuos con este tipo de leucemia, sin embargo, se ha asociado a un amplio espectro de otras enfermedades autoinmunes y afecciones hematológicas incluyendo anemia hemolítica, aplasia pura de glóbulos rojos y neutropenia, que conducen a infecciones bacterianas recurrentes. Se presenta a continuación una paciente de 72 años con antecedentes de leucemia de células T grandes granulares y manifestaciones compatibles con artritis reumatoidea, que intercurre con un Síndrome de Evans grave con buena respuesta inicial y sostenida a gammaglobulina, corticoterapia, y rituximab.

Abstract Large granular T-cell leukemia is a rare cytotoxic lymphocyte disorder. These cells play an integral role in the immune system and are divided into 2 lineages: CD3 T positive and natural killer. Its proliferation and uncontrolled cytotoxicity can generate autoimmunity or malignancy. Rheumatoid arthritis is the most common autoimmune disease in individuals with this type of leukemia, however, it has been associated with a wide spectrum of other autoimmune diseases and hematological conditions including hemolytic anemia, pure red blood cell aplasia, and neutropenia, leading to recurring bacterial infections. The following is a case of a 72-year-old female with a history of large granular T-cell leukemia and manifestations compatible with rheuma toid arthritis, which occurs with a severe Evans syndrome with a good initial and sustained response to gamma globulin, corticosteroid therapy, and rituximab.

Acquired factor XIII deficiency in patients under therapeutic plasma exchange: A poorly explored etiology.

INTRODUCTION: Factor XIII (FXIII) deficiency may cause bleeding under certain clinical circumstances. Therapeutic plasma exchange (TPE) may lead to a transient deficiency. OBJECTIVES: To describe the clinical evolution of patients with acquired FXIII deficiency secondary to TPE. METHODS: We respectively studied a cohort of consecutive patients from 2014 to 2019 who were treated with TPE with FXIII levels <50%. The FXIII was measured after the start of the TPE course, on days between the TPE sessions, due to suspected acquired deficiency. All TPE were performed using continuous flow cell separator. In all cases, the initial replacement fluid applied was albumin. Apheresis procedures were held at 24to 48 hours intervals. RESULTS: Eighteen patients were included, 13 of them were recipients of kidney transplants. The main TPE prescription was humoral rejection. Median FXIII at diagnosis (measured on days between sessions of the TPE course) was 19%(IQR17-25). The median of apheresis procedures before measurement of FXIII was 3(IQR2-4). Among the total cohort, 10 patients suffered hemorrhages. None of the patients without history of kidney transplants had bleeding (n = 5), however, 10/13 with kidney transplants did. Five kidney transplant patients received therapy with FXIII concentrate because of life-threatening bleeding. In all cases, the bleeding stopped within the first 24 hours. All patients had their FXIII levels measured again after finishing the TPE course, with normal results. CONCLUSIONS: TPE is an under-diagnosed cause of acquired FXIII deficiency since routine coagulation tests remain unaltered. It might cause major bleeding, particularly in patients with a recent history of surgery like kidney transplants.

Nonimmune-acquired factor XIII deficiency: a cause of high volume and delayed postoperative hemorrhage.

: Factor XIII (FXIII) levels may decrease because of surgical consumption. Acquired FXIII deficiency could be a cause of postoperative hemorrhage usually underdiagnosed in clinical practice. To determine the diagnosis confirmation rate of acquired FXIII deficiency in postsurgical patients with clinical suspicion and to compare the characteristics and evolution of patients with or without FXIII deficiency. We performed a retrospective cohort study, which included 49 inpatients who were attended at our university hospital from 2014 to 2018 with suspicion of acquired FXIII deficiency because of disproportionate postoperative hemorrhage. FXIIIA levels less than 50% was considered a deficiency. Persistence of bleeding for more than 48 h, drop in hematocrit points, red blood cells transfused units, hemoglobin levels 12-36 h after bleeding, and time elapsed from the procedure to the bleeding were assessed as outcome variables. Logistic regression was employed for both univariate and multivariate analyses. Of the 49 patients included, 27(55%) had FXIII deficiency, with a median level of 34% [interquartile range (IQR) 19-42]. Abdominal surgery was the most common [n = 21 (43%)]. All patients had routine coagulation tests within the hemostatic range. FXIII deficiency was associated with a drop of more than 4 points in hematocrit [OR 59.69 (95% CI 4.71-755.30)], red blood transfused units >2 [OR 45.38 (95% CI 3.48-590.65)], and delayed bleeding >36 h after surgery [OR 100.90 (95% CI 3.78-2695.40)]. Plasma-derived FXIII concentrate was administered to eight patients with life-threatening bleeding with resolution within 24 h. Only one deficient patient died from bleeding. FXIII levels were measured 15 days after diagnosis or more in 20 out of 27 deficient patients, with normal results. Acquired FXIII deficiency may be a frequent underdiagnosed entity that should be considered when high-volume and delayed postoperative hemorrhage is present in patients with hemostatic routine coagulation test results.

Hidronefrosis como causa de poliglobulia secundaria / Hydronephrosis as a cause of secondary polyglobulia

La policitemia primaria es producida por una mutación adquirida o heredada en las células progenitoras de los glóbulos rojos, mientras que la poliglobulia secundaria está relacionada con un aumento de la eritropoyetina sérica como respuesta a la hipoxia tisular o a la producción autónoma tumoral. Hace más de medio siglo que se conoce que la hidronefrosis puede actuar como una rara causa de eritrocitosis debido al aumento de producción de eritropoyetina por un riñón que censa una disminución de oxígeno, mecanismo también observado en la estenosis de la arteria renal y en los quistes renales. Se describe a continuación el caso de un paciente de 38 años con poliglobulia atendido en el Hospital Italiano de San Justo (Argentina), que presenta como hallazgo una hidronefrosis unilateral severa y cuya resolución quirúrgica a través de una nefrectomía revierte el cuadro hematológico de base. (AU)

Primary polycythemia is produced by an acquired or inherited mutation in progenitor cells of red blood cells, while secondary polyglobulia is related to an increase in serum erythropoietin in response to tissue hypoxia or autonomous tumor production. Since the middle of the twentieth century, the hydronephrosis is known to be a rare etiology of secondary polycythemia, with increased erythropoietin production caused by diminished oxygen sensing by the kidney, also seen in renal artery stenosis and kidney cysts. We describe a case of a 38 year old patient with polycythemia studied in the "Hospital Italiano de San Justo" (Argentina) that presented an incidental severe unilateral hydronephrosis, and nephrectomy was carried out as a final resolution of the hematological disorder. (AU)